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. 2022 Feb 28;19(2):137–151. doi: 10.11909/j.issn.1671-5411.2022.02.006

Table 1. Main histological subtypes of fulminant myocarditis.

Subtype of fulminant myocarditis Pathology Incidence Etiology Clinical presentation/Diagnosis Treatment
Lymphocytic myocarditis Small mononuclear cells (CD3+ T lymphocytes) The most frequent
histological subtype
Viruses
Drugs/toxins
Autoimmune
Wide range of presentations
The most frequent subtype in asymptomatic patients
Frequently self-limited
In fulminant myocarditis,
mechanical circulatory support as supportive care
No clear evidence of immunosuppressants
Eosinophilic myocarditis Eosinophilic infiltrate Rare: unknown Hypersensitivity (antibiotics, clozapine, carbamazepine)
Churg-Strauss
Hypereosinophilic syndrome
Parasitic infections (Toxocara canis)
From asymptomatic to fulminant myocarditis or Loeffler cardiomyopathy
Fever, skin rash if hypersensitivity
Peripheral eosinophilia not always present
Frequent intraventricular thrombosis
Identifying cause
High-dosing corticosteroids +/− cyclophosphamide, albendazole, imatinib, azathioprine or methotrexate
Mechanical circulatory support (mainly in hypersensitivity and idiopathic forms)
Giant cell myocarditis Large multinuclear cells in the absence of well-formed granuloma
Degranulated eosinophils
Uncommon Unknown
Autoimmune disorders frequently associated (25%)
Young, healthy adults
Severe heart failure, refractory cardiogenic shock
Frequent arrhythmic disturbances (atrioventricular block, ventricular tachycardia, ventricular fibrillation)
Aggressive, combined, immunosuppressants treatment: cardiogenic shock + cyclosporine-based treatment
Rabbit anti-thymocyte immunoglobulin
Muromunab, azathioprine: second-line treatment
Frequent need of mechanical circulatory support
Immune checkpoints inhibitors-checkpoint myocarditis Similar to a high-grade cardiac rejection: T cell mediated injury Less than 1% of treated patients Immune checkpoints inhibitors (nivolumab, pembrolizumab) Life-threatening arrhythmic disturbances (atrioventricular block, refractory ventricular tachycardia), multiorgan failure
Early presentation after initiation of immune checkpoints inhibitors
(< 6 weeks)
Possible other organ involvement (liver, lung, etc)
Withdrawal of the drug
High dosing of corticosteroids
Mechanical circulatory support